Proteus syndrome and immunodeficiency.

Hodge, D; Misbah, S A; Mueller, R F; Glass, E J; Chetcuti, P A · Arch Dis Child · 2000

case_report · Level V

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Abstract

A 10 year old boy with Proteus syndrome presented with a pericardial effusion of unknown aetiology. Immunological investigation revealed low serum IgG and IgA, accompanied by low levels of specific antibodies to pneumococcal and haemophilus type B polysaccharides. Circulating lymphocyte surface marker profile revealed T and B cell lymphopenia. This is the first report of hypogammaglobulinaemia occurring in the Proteus syndrome.

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